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Commentary: Heterogeneity of respiratory disease in children and young adults with sickle cell disease
  1. Robyn T Cohen1,
  2. Elizabeth S Klings2
  1. 1Department of Pediatrics, Boston University School of Medicine, Boston, Massachusetts, USA
  2. 2Department of Medicine, The Pulmonary Center, Boston University Medical Campus, Boston, Massachusetts, USA
  1. Correspondence to Dr Elizabeth S Klings, Department of Medicine, The Pulmonary Center, Boston University School of Medicine, Boston MA 02118, USA ; klingon{at}bu.edu

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Sickle cell disease (SCD) affects 300 000 children born worldwide annually. While classically thought of as a genetic haemoglobinopathy, complications impact nearly every vascular bed leading to progressive organ damage and accelerated mortality. Pulmonary complications of SCD are among the most common causes of morbidity and mortality and affect every structure of the lungs. Abnormal pulmonary function is often one of the earliest indicators of lung disease, yet our understanding of the clinical significance of this finding is poor. 

Prior studies suggest several consistent themes: (1) while most children with SCD have lung function within the normal range,1 2 spirometry is, on average, reduced compared with healthy …

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